Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review

Congenital adrenal hyperplasia (CAH) refers to group of inherited diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21-hydroxylase deficiency. Testicular adrenal rest tumors (TARTs) are an important complication of CAH, which probably develop from ectopic remnant...

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Autores principales: Hiba Hassan Ali, Alaa Samkari, Haitham Arabi
Formato: article
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Publicado: Thieme Medical and Scientific Publishers Pvt. Ltd. 2013
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Acceso en línea:https://doaj.org/article/8f9b9c82f8d34227b83caedba882c641
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spelling oai:doaj.org-article:8f9b9c82f8d34227b83caedba882c6412021-12-02T17:59:10ZTesticular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review2231-07702249-446410.4103/2231-0770.112789https://doaj.org/article/8f9b9c82f8d34227b83caedba882c6412013-01-01T00:00:00Zhttp://www.thieme-connect.de/DOI/DOI?10.4103/2231-0770.112789https://doaj.org/toc/2231-0770https://doaj.org/toc/2249-4464Congenital adrenal hyperplasia (CAH) refers to group of inherited diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21-hydroxylase deficiency. Testicular adrenal rest tumors (TARTs) are an important complication of CAH, which probably develop from ectopic remnants of intra-testicular adrenal tissue stimulated by Adrenocorticotropic hormone (ACTH) hypersecretion. These lesions are typically located within the rete testis and are bilateral, synchronous, nodular and multiple. TART usually, but not always, responses to suppressive medical therapy. TART leads to testicular structural damage, spermatogenesis disorders, infertility and most importantly, mass-forming lesions that could be mistaken for Leydig cell tumor (LCT). The later has a significantly different behavior with up to 10% of being malignant. Nowadays, due to advances in diagnosing and treating CAH, mass-forming TART is rarely encountered. As a result, there is the paucity in the medical literature regarding its features from pathological perspective. We herein present a case of mass-forming TART and we discuss the clinical, radiological, and morphological features as well as the major differential diagnosis of this rare lesion.Hiba Hassan AliAlaa SamkariHaitham ArabiThieme Medical and Scientific Publishers Pvt. Ltd.articleleydig cell tumortesticular adrenal rest tumortesticular massMedicineRENAvicenna Journal of Medicine, Vol 03, Iss 01, Pp 15-19 (2013)
institution DOAJ
collection DOAJ
language EN
topic leydig cell tumor
testicular adrenal rest tumor
testicular mass
Medicine
R
spellingShingle leydig cell tumor
testicular adrenal rest tumor
testicular mass
Medicine
R
Hiba Hassan Ali
Alaa Samkari
Haitham Arabi
Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
description Congenital adrenal hyperplasia (CAH) refers to group of inherited diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21-hydroxylase deficiency. Testicular adrenal rest tumors (TARTs) are an important complication of CAH, which probably develop from ectopic remnants of intra-testicular adrenal tissue stimulated by Adrenocorticotropic hormone (ACTH) hypersecretion. These lesions are typically located within the rete testis and are bilateral, synchronous, nodular and multiple. TART usually, but not always, responses to suppressive medical therapy. TART leads to testicular structural damage, spermatogenesis disorders, infertility and most importantly, mass-forming lesions that could be mistaken for Leydig cell tumor (LCT). The later has a significantly different behavior with up to 10% of being malignant. Nowadays, due to advances in diagnosing and treating CAH, mass-forming TART is rarely encountered. As a result, there is the paucity in the medical literature regarding its features from pathological perspective. We herein present a case of mass-forming TART and we discuss the clinical, radiological, and morphological features as well as the major differential diagnosis of this rare lesion.
format article
author Hiba Hassan Ali
Alaa Samkari
Haitham Arabi
author_facet Hiba Hassan Ali
Alaa Samkari
Haitham Arabi
author_sort Hiba Hassan Ali
title Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
title_short Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
title_full Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
title_fullStr Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
title_full_unstemmed Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review
title_sort testicular adrenal rest "tumor" or leydig cell tumor? a report of a challenging case with literature review
publisher Thieme Medical and Scientific Publishers Pvt. Ltd.
publishDate 2013
url https://doaj.org/article/8f9b9c82f8d34227b83caedba882c641
work_keys_str_mv AT hibahassanali testicularadrenalresttumororleydigcelltumorareportofachallengingcasewithliteraturereview
AT alaasamkari testicularadrenalresttumororleydigcelltumorareportofachallengingcasewithliteraturereview
AT haithamarabi testicularadrenalresttumororleydigcelltumorareportofachallengingcasewithliteraturereview
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